Cystic echinococcosis (CE) is a widely endemic helminthic disease caused by infection with metacestodes (larval stage) of the Echinococcus granulosus tapeworm and transmitted by dogs. E. granulosus are common parasites in certain parts of the world, and are present on every continent with the exception of Antarctica. As a result, a large number of people are affected by CE. In humans, the disease is characterized by slowly growing cyst commonly occurring in liver and lungs. Clinical features are mainly right upper quadrant pain, feeling of lump and enlarged tender liver. The cyst may be complicated by infection or rupture and may lead to anaphylactic reaction. The diagnosis depends on clinical suspicion. They appear in two ways as general (systemic) symptoms, and local symptoms based on the site and organ on which the larva settles. While cysts sometimes recover spontaneously, more severe clinical presentations are observed in immunosuppressed individuals. Ultrasonography supported by serology is the main diagnostic modality. The treatment varies from surgical intervention to minimally invasive treatments (percutaneous drainage) or medical therapies. Surgery is still the best treatment modality. Percutaneous drainage of the cyst is a good option in selected cases. New sensitive and specific diagnostic methods and effective therapeutic approaches against echinococcosis have been developed in the last 10 years.
Congenital anterior urethral diverticulum (CAUD) is an uncommon condition in children. We describe 2 patients of CAUD who presented with ventral penile swelling; in one, the site of swelling was just proximal to corona which is quite rare. The diagnosis was made on USG and MCU. Both patients had normal renal function. Open diverticulectomy and primary repair was done in both patients.
HighlightsTrichobezoar leading to Rapunzel syndrome is an extremely rare entity.The clinical presentation is usually vague and non-specific but psychiatric illness is the usual association.Treatment is mainly surgical because of delayed presentation in majority of the cases.Treatment should focus on prevention of recurrence, apart from removal of the mass.So, in addition to the acute surgical treatment, parental counselling, neuropsychiatric treatment, follow-up and behavioural therapy of the patient should be continued to prevent recurrence.
HighlightsAdenoid cystic carcinoma (ACC) of the breast is a rare type of primary breast cancer accounting for less than 0.1% of all primary breast cancers.Unlike other triple negative breast cancers that are associated with poor prognosis, ACC has an overall excellent prognosis.Mammographically, these tumors may appear as asymmetric densities or irregular masses. Sonographically, they appear as well-defined, irregular, heterogeneous, or hypoechoic masses. Nonetheless, the radiographic findings are non-specific and can be misdiagnosed as benign lesions. Subsequently, it could be challenging for a radiologist to make the correct diagnosis of carcinoma without histologic confirmation.Due to the rarity of the tumor and large variations in the patterns of practice, guidelines for treatment have not been established. Because of these distinct clinicopathologic features that set it apart from the other triple negative breast cancers, an understanding of ACC of the breast is essential for surgical pathologists, breast surgeons, and oncologists.
HighlightsSPT is rare, but treatable pancreatic tumor.While clinical signs and symptoms are relatively nonspecific, characteristic findings on imaging and histology separate these tumors from the more malignant pancreatic tumors.The identification of a large bulky pancreatic tumour in a child or woman should raise suspicions of solid pseudopapillary tumour of the pancreas.Surgical excision offers the best chance for cure and should always be attempted irrespective of the magnitude of resection involved.Patients with SPT have an excellent prognosis after surgical excision. The prognosis is favorable even in the presence of distant metastasis.
Background
Wilms’ tumor (WT) is the most frequently occurring paediatric renal tumor and is one of the most treatment-responsive tumors. A tumor-suppressor gene and other genetic abnormalities have been implicated in its etiology. In addition, patients with many congenital anomalies, such as Beckwith-Wiedemann syndrome, WAGR syndrome and Denys-Drash syndrome, have an increased risk of WT.
Methods and results
Two large collaborative groups – National Wilms Tumor Study Group (NWTSG)/Children's Oncology Group (COG) and The International Society of Paediatric Oncology (SIOP) have laid down the guidelines for standardized treatment of WT, though differing in the diagnostic and therapeutic approach. The major difference in the two guidelines is the timing of surgery: SIOP recommends using preoperative chemotherapy and NWTSG/COG prefers primary surgery before any adjuvant treatments. Both these groups currently aim at intensifying treatment for patients with poor prognosticators while appropriating the therapy to reduce long-term complications for those with favourable prognostic features. As the survival rate has now reached 90%, the primary objectives of the physician are to perform nephron-sparing surgery in selected cases and to reduce the dosage and duration of chemotherapy and radiotherapy in appropriate cases. The purpose of this review is to present current standards of diagnosis and treatment of WT around the world.
Conclusion
Further studies in future should be done to highlight the use of chemotherapy and radiotherapy under risk-stratified strategies. Further improvement in survival of these children can only be achieved by increasing awareness, early recognition, appropriate referral, and a multidisciplinary approach.
Retrocaval ureter is one of the very rare congenital anomalies. We report a case of retrocaval ureter in a 6-year-old girl who presented with right flank pain and hydronephrosis. The diagnosis was made on intravenous urography which showed typical “J” shape deformity in the proximal dilated ureter with moderate hydronephrosis. CT scan delineated the course of ureter. The patient was operated and findings were confirmed. The ureter was transected near the pelvis and a pyeloureteric anastomosis with pre-caval transposition of the ureter was performed and the patient was discharged in fair health.
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